Stiefel Dermatology Review

Front 1

Name the free sebaceous glands

Back 1

Montgomery's areolar tubercles
Fordyce's condition
Meibomian glands - chalazion
Glands of Zeis - hordeolum
Tyson's glands

Front 2

What is the irritant in Garlic and Onions?

Back 2

Diallyldisulfide

Front 3

Ab to what in Bullous Lupus?

Back 3

Ab to Type VII collagen (in sublamina densa)
DIF shows linear IgG, IgM, IgA and C3

Front 4

MW of Plakoglobin?

Back 4

Found in both adherens and desmosomes
85kDa

Front 5

Irritant found in buttercups?

Back 5

Protoanemonins

Front 6

Irritant found in dumb caine (Dieffenbachia) and daffodils?

Back 6

Calcium oxalate

Front 7

Irritant found in the croton plant?

Back 7

Phorbol esters

Front 8

Name the TH2 cytokines

Back 8

IL4, IL5, IL6, IL10, IL13

Front 9

In porphyria pathway, what is the rate limiting step?

Back 9

ALA synthase
First and rate-limiting step in the heme synthesis pathway
Occur in the mitochondria and allows for conversion of Succinyl coA + glycine to deta aminolevilinic acid

Front 10

What is dimethylglyoxime?

Back 10

chemical that when applied to metals containing nickel will turn pink

Front 11

What is Dowicil?

Back 11

formaldehyde-releasing preservative

Front 12

What is allergen in primrose (Primula obconica)?

Back 12

Primin, a type of quinone

Front 13

Name some formaldehyde-releasing preservatives

Back 13

Germail, quaternium 15 (dowicil 200), DMDM hydantoin

Front 14

What is Methylchloroisothiazolinone?

Back 14

aka Kathon CG
Is a preservative

Front 15

"Oriental" tiger balm cross reacts with what?

Back 15

Balsam of Peru

Front 16

"Bavarian" tiger balm cross reacts with what?

Back 16

Rosin

Front 17

Name drugs that can cause cicatricial pemphigoid

Back 17

Clonidine
sulfa drugs
thiol containing drugs

Front 18

DH is assoc with what HLA?

Back 18

HLA DR3 and DQ2

Front 19

Drug causes of Pseudoporphyria

Back 19

NSAIDs,
Lasix, TCN, accutane, amiodarone & tanning beds

Front 20

Leiner's disease

Back 20

aka erythroderma desquamativum
assoc with deficient C5 and possible C3
clinical: babies w/ diarrhea, infections (spesis), anemia, and generalized seborrheic dermatitis like rash

Front 21

Blue lunulae are associated with what diseases?

Back 21

Argyria
Wilson's Disease
Hereditary acrolabial telangiectasia
Paronychia
Phenolphthalein
Quinacrine
Topical bichloride
Mercury exposure

Front 22

Yellow nail syndrome is assoc with what disease?

Back 22

Lymphedema and bronchiectasis -- are most common

Can also see yellow nails with RA, thyroid disease, malignancies, chronic sinusitis or bronchitis.


*(note: is also associated with use of D-penicillamine for RA treatment)*

Front 23

What diseases are associated with dorsal pterygium?

Back 23

LP
Acrosclerosis
Onychotillomania
Lesch-Nyhan syndrome
Chronic GVHD
SJS/TEN
Cicatricial pemphigoid

Front 24

What syndrome is characterized by broad thumbs and nails (aka brachyonychia)?

Back 24

Rubenstein-Taybi
(mutations in CREB-binding protein)

Front 25

Name the disease:
mental and physical retardation
convulsions
episodic unconsciousness
liver enlargement
skin lesions
dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red

Back 25

argininosuccinic aciduria

Front 26

ventral pterygium is assoc with what disease?

Back 26

systemic sclerosis

(vs. dorsal pterygium seen in LP, cicatricial pemphigoid, SJS/TEN, chronic GvHD)

Front 27

What is:
COIF Syndrome?
DOOR Syndrome?
Coffin-Siris Syndrome?

Back 27

COIF: Congenital Onychodysplasia of the Index Finger

DOOR: Deafness, Congenital Onychodystrophy Recessive Form

Coffin-Siris: has nail onychodysplasia or aplasia (usu of the fifth finger or toe), coarse facies including bushy eyebrows, scant scalp hair, full lips, and microcephaly, mental/growth deficiency, short distal phalanges and other abnormalities

Front 28

Name the syndrome:
cutis marmorata
hirsutism
synophrys
MR
short stature
GU abnormalities
hearing loss
congenital heart defects

Back 28

Cornelia de Lange Syndrome
(sporadic mutation in nipped-Beta-like gene

Front 29

Name an example of 'Apparent Leukonychia"

Back 29

Muehrcke's lines (from hypoalbuminemia)

Apparent leukonychias disappear with pressure (problem with the nail bed not the nail plate)

Front 30

Bjornstad's syndrome has what key features?

Back 30

Pili torti
Deafness

Front 31

Name the syndrome & defect:
wooly hair
diffuse PPK
Right sided heart failure

Back 31

Naxos -- plakoglobulin gene

Front 32

Name the syndrome:
poliosis
uveitis
deafness
vitiligo

Back 32

Vogt-Koyanagi-Harada Syndrome

Front 33

Defect in Hay-Wells? Another name for it? Other clinical findings?

Back 33

defect: P63
aka Ankyloblepharon-Ectodermal dysplasia-Clefting
Findings: wiry, sparse hair or alopecia, ankyloblepharon, PPK, partial anhidrosis, cleft lip, palate, absent and dystrophic nails

Front 34

Defect in EEC (ectrodactyly-ectodermal dysplasia-clefting)?

Back 34

P63

Front 35

Gene associated with Trichodentooseous syndrome?

Back 35

DLX3 (distal-less homeobox-3) gene

(clinical picture: curly hair that straightens in the 2nd-3rd decade, enamel hypoplasia, dental pits, and increased bone density)

Front 36

Defect in Cartilage-Hair hypoplasia syndrome?

Back 36

RMRP (RNA component of RNase MRP)

Front 37

Gene mutation in Tricho-rhino-phalangeal syndrome?

Back 37

TRPS1 (a putative zinc finger transcription factor); AD inheritance

Front 38

Name the disease
nail hypoplasia
absent or hypoplastic patella
posterior iliac horns
glaucoma
hyperpigmentation of the pupillary margin (or Lester Iris)
glomuerulonephritis
Nephrotic syndrome

Back 38

Nail-Patella Syndrome

Front 39

Name the progestins with low intrinsic androgenic properties.

Back 39

Desogestril, norgestimate, gestodone

Front 40

Name some drugs that have been known to cause Sweet's Syndrome

Back 40

granulocyte colony-stimulating factor (G-CSF), all-trans retinoic acid, hydralazine, cabamazepine, levonorgestrel/ethinyl estradiol, TMP/SMZ, MCN

Front 41

Leiner's Disease

Back 41

Disease associated with deficiency in C5 and possibly C3.
Babies are prone to diarrhea, infections (sepsis), anemia, and a generalized seborrheic dermatitis-like rash.

Front 42

What is the most common cause of paraneoplastic pemphigus?

Back 42

Non-Hodgkin's lymphoma
(Castleman's disease is the most common in children with paraneoplastic pemphigus)

Others: CLL, sarcoma, thymoma

Front 43

Antibodies to what proteins have been found in Stevens-Johnson?

Back 43

Antibodies to desmoplakin I and II have been found.

Front 44

Solar urticaria has been associated with what other disorders?

Back 44

Rare cases have been associated with EPP, Lupus erythematosus (LE) and with certain drugs.

Front 45

Name the branch of the ICA that supplies the face

Back 45

Ophthalmic

Front 46

Name the branches of the Ophthalmic artery

Back 46

Supraorbital
Supratrochlear
Palpebral
Dorsal nasal
Lacrimal

Front 47

What Margins are adequate?
BCC
SCC
Atypical Nevi
Melanoma in situ
Invasive melanoma (<1mm)

Back 47

BCC 4mm (if <2cm), 10mm if >2cm
SCC 4-6mm if <2cm, 10mm if >2cm
Atypical Nevi 2-3mm
Melanoma in situ - 5mm
Invasive Melanoma (<1mm) - 1 cm and deep fat (to fascia layer)

Front 48

Name the branches of the Facial Artery

Back 48

Inferior labial
Superior labial
Angular

Front 49

Name the branches of the Maxillary Artery

Back 49

Infrarobital
Buccal
Inferior alveola (mental)

Front 50

How much of a wedge excision can you take from lip?

Back 50

1/3 of lip

Front 51

Anastamoses of the ICA and ECA is which artery?

Back 51

Angular artery (continuation of Facial artery and Infraorbital artery) with Supratrochlear (ICA)

Front 52

What nerves supply the lateral neck and posterior scalp?

Back 52

Cervical Plexus

Front 53

Name the major arteries off of the ECA that supply the face

Back 53

Facial
Maxillary
Superficial temporal
Posterior Auricular
Occipital
(Ophthalmic supplies face but comes off the ICA!)

Front 54

What nerves run thru Erb's Point?

Back 54

Erb's Point = near the mid-posterior SCM
Greater auricular
Lesser occipital
Spinal accessory

Front 55

What gene translocation is found in DFSP?

Back 55

T(17;22)(q22;q13): fusion of two genes: collagen type 1 alpha 1 (COL1A1) and platelet-derived growth factor B-chain (PDGFB)

Front 56

What CD markers are lost on MF cells?

Back 56

MF sometimes lose CD5 as well as CD7; CD4 positive.

Front 57

What CD is a marker for B-cells?

Back 57

CD20

Front 58

Chromagranin stains what type of cells?

Back 58

neuroendocrine cells, Merkel cell carcinomas and eccrine cells

Front 59

Brooke-Spiegler syndrome

Back 59

Multiple cylindromas
multiple trichoepitheliopas
multiple spiradenomas

Front 60

Alagille syndrome

Back 60

Arteriohepatic dysplasia and nevus comedonicus

Front 61

Nicolau-Balus syndrome

Back 61

multiple eruptive syringomas, milia, and atrophoderma vermiculata

Front 62

Schopf's syndrome

Back 62

multiple apocrine hidrocystomas
palmar-plantar keratoderma
teeth abnormalities (hypodontia)
onychodystrophy

Front 63

Lhermitte-duclos disease

Back 63

manifestation of cowden's:
proliferation in the cerebellum (dysplastic gangliocytoma) with macrocephaly

Front 64

Name diseases with multiple pilomatricomas

Back 64

Rubinstein-Taybi
Gardners
Turner's
Sarcoidosis
Sternal cleft and coagulation defects
Myotonic dystrophy (steinert's)

Front 65

Birt-Hogg-Dube syndrome

Back 65

AD, mutation in folliculin;
multiple small hamartomas of mesodermal component of hair discs: trichodiscomas, acrochordons, fibrofolliculomas, collagenomas, lipomas and/or oral fibromas, and renal tumors
trichodiscomas

Front 66

Eccrine glands are NOT found where?

Back 66

vermillion of lips
glans
labia minora
nail beds
inner prepuce

Front 67

Neuron-specific enolase is positive in what conditions?

Back 67

Merkel Cell Carcinomas
Carcinoid tumors
Malignant Melanoma

Front 68

Multiple/eruptive syringomas seen in what condition? Clear cell change in syringomas seen in what condition?

Back 68

Down's = multiple syringomas
Diabetes = clear cell change

Front 69

Name the diseases related to mutations in Gap Junctions

Back 69

Connexin 26 = Vohwinkels, KID, PPK with deafness; Connexin 30.3 & 31 = Erythrokeratoderma variabilis; Connexin 30 = hidrotic ectodermal dysplasia

Front 70

Mucosal melanomas tend to have what mutation?

Back 70

Activating mutation in KIT

Front 71

GNAQ mutations are seen in which types of melanomas?

Back 71

Uveal melanomas and blue nevi

Front 72

Retinoids upregulate the transcription of which collagens?

Back 72

Collagen 1 & 7

Front 73

What growth factors are released by platelets to promote new tissue growth?

Back 73

PDGF, TGF-alpha and TGF-beta (platelets also release ADP, but this is not a growth factor)

Front 74

What growth factors are released by monocytes in wound healing?

Back 74

FGF, IL-1, PDGF, TGF-alpha, and TGF-beta

Front 75

What do mast cells release in wound healing?

Back 75

Neutrophil chemotactic factor

Front 76

What activates apocrine glands?

Back 76

Epinephrine and norepinephrine; these glands operate by decapitation secretion

Front 77

Where are apocrine glands located?

Back 77

Axillae, anogenital, moll's glands (eyelids), mammary glands of the breast and ceruminous glands of the external auditory canal.

Front 78

Eccrine glands respond to what type of stimulation?

Back 78

cholinergic sympathetic nerves (mediated by acetocholine)

Front 79

Sebaceous glands respond to what stimulation?

Back 79

Androgen-responsive holocrine glands

Front 80

At what EGA are all layers of the keratinized epidermis identifiable?

Back 80

24 weeks

Front 81

What are the main constituents of human sebum?

Back 81

Squalene, cholesterol, cholesterol esters, triglycerides and wax esters.

Front 82

How is human sebum different from internal organ lipids?

Back 82

Human sebum is distinguished by presence of wax esters and squalene.

Front 83

What are the mast cell mediators?

Back 83

Mast cells produce and store: histamine, heparin, tryptase, chymase, carboxypeptidase, neutrophil chemotactic factor and eosinophilic chemotactic factor of anaphylaxis. They release (without storing) - growth factors, cytokines, and leukotrienes.

Front 84

Describe Stage I or the Emergent Care Phase.

Back 84

Begins at the time of injury and concludes with the restoration of capillary permeability, which typically reverses 48 to 72 hours following an injury.

Characterized by fluid shift from intravascular to interstitial and shock. Focus on care is to preserve vital organ functioning.

Expect to administer large volumes of fluid in this face.
Parkland (4ml x TBSA x kg) = 24hr fluids

Goals: establish airway
save life
minimize disability
prepare for definitive care in the next 24-36hrs

Front 85

What is the most abundant acid that composes the odor from apocrine secretions?

Back 85

3-methyl-2-hexenoid acid

Front 86

Warts in renal transplant patients have show increased expression of what keratin?

Back 86

K13

Front 87

Where are Krause end bulbs found?

Back 87

Mucocutaneous receptors found on the glans penis, clitoris, labia minora, perianal area, and vermillion border of the lips.

Front 88

DIF is of no value in which diagnosis?

Back 88

Scleroderma, morphea, and neonatal lupus.

Front 89

What EGA does epidermal stratification occur?

Back 89

8 weeks

Front 90

Most common site for carcinoid tumor?

Back 90

appendix

Front 91

Name a newly reported side effect of Imiquimod

Back 91

eruptive epidermoid cysts (aldara is TLR7)

Front 92

What drug can cause a lupus-like syndrome and hepatitis?

Back 92

Minocycline

Front 93

What drug can have the side effect of vascular leak syndrome?

Back 93

Denileukin difitox (engineered protein combining IL-2and Diphtheria toxin)

Front 94

What is it called when potassium iodide may lead to inhibited thyroid hormone synthesis from excess iodides (blocks organic iodides from binding in the thyroid)?

Back 94

Wolff-Chaikoff effect

Front 95

Thalidomide is most associated with what adverse effect?

Back 95

Most common neuropathy is mild proximal muscle weakness with symmetric sensory painful paresthesias of the distal extremities with accompanying lower limb sensory loss.

Front 96

What are common sites for chloracne?

Back 96

malar cheek, post-auricular scalp, and scrotum

Front 97

When is maximal vasoconstriction after infiltration of lidocaine with epi?

Back 97

15 minutes

Front 98

What is the growth medium for Neisseria gonorrhea?

Back 98

Chocolate agar in 10% CO2 (Thayer-martin, Martin-Lewis, or New York City medium will also grow N. gonorrhea);

Front 99

What is needed to culture out hemophilus influenza?

Back 99

Agar supplemented with heme (x-factor) and nicotinamide (v-vactor)

Front 100

What type of agar is used to identify hemolytic strains of strep and staph?

Back 100

Sheep's blood agar